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Amyloid deposition associated with generalized morphea‐like scleroderma

European Journal of Dermatology. Volume 13, Number 5, 509-11, September 2003, Clinical report

Summary

Author(s) : Koji SUMI Toshiyuki YAMAMOTO Hiroo YOKOZEKI Kiyoshi NISHIOKA , Department of Environmental Immunodermatology, Tokyo Medical and Dental University Postgraduate School, Yushima 1‐5‐45, Bunkyo‐ku, Tokyo, Japan .

Summary : A 62‐year‐old man with amyloid deposition associated with generalized morphea‐like scleroderma is described. He had been occupationally exposed to organic solvents. Physical examination showed sclerosis of fingers, forearms, and trunk. Erythema was noted on the border of patchy sclerotic plaques on his chest and back. In addition, firm miliary, keratotic papules were found on the lateral forearms. Amyloid deposition was demonstrated by Congo‐red stain at papillary layers of overlying sclerotic dermis in the biopsied specimen taken from the left forearm. As far as we know, amyloid deposition associated with generalized morphea‐like scleroderma has not been reported until now.

Keywords : amyloid, generalized morphea‐like scleroderma, organic solvents

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ARTICLE

Auteur(s) : Koji SUMI Toshiyuki YAMAMOTO Hiroo YOKOZEKI Kiyoshi NISHIOKA

Department of Environmental Immunodermatology, Tokyo Medical and Dental University Postgraduate School, Yushima 1‐5‐45, Bunkyo‐ku, Tokyo, Japan

Reprints: K. Sumi Fax: (+81) 3‐5803‐0143 E‐mail: kojidermtmd.ac.jp

Article accepted on 10\7\2003

Key words: Generalized morphea‐like scleroderma was first reported by Yamakage and Ishikawa [1, 2]. The clinical features of generalized morphea‐like scleroderma are different from those of systemic sclerosis. Generalized morphea‐like scleroderma clinically involves the upper arms, lateral forearms, lumbar area, and back, which shows symmetrical and lesional sclerosis with pigmentation and depigmentation intermingled within the sclerotic plaques as well as redness on the borders. Frequently, there is a history of exposure to organic solvents [2]. Our patient presented not only the clinical features of generalized morphea‐like scleroderma but also lesional miliary papules on the lateral aspects of sclerotic forearms. Skin biopsies from the forearm showed amyloid deposition in the papillary dermis, which was identified by Congo‐red stain.

Case report

A 62‐year‐old Japanese man was referred to our hospital complaining of dyspnea and coldness of his limbs. He had no particular past history nor family history. His job was sale of building materials and organic solvents at his office. Three years ago, he noticed itching of the head and face, sclerosis of the back, lumbar area and superior limbs, and Raynaud‘s phenomenon. Furthermore, he had been conscious of dyspnea for one year.

Physical examination showed comparatively‐circumscribed shiny plaques on the cervix, lumbar area, back and lateral upper arms. In the V‐neck zone, redness and small depigmented patches were diffusely recognized (Fig.1). Diffuse sclerosis and pigmentation could be observed on his head and face. In addition, miliary papules were found at the lateral aspects of sclerotic forearms (Fig.2).

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Laboratory studies showed that the anti‐nuclear antibody was positive (1:320, speckled pattern) and serum KL‐6 levels were 3540 U\ml (normal; < 500 U\ml). The anti‐ribonucleoprotein (RNP) antibody, the anti‐Scleroderma‐70 (Scl‐70) antibody, the anti‐SS‐A antibody, and the anti‐SS‐B antibody were negative. The rheumatoid factor was 245 IU\ml (normal: < 20 IU\ml). Serum immunogloblin E levels were 2745 IU\ml (normal: < 250 IU\ml). The arterial oxygen tension (PaO2) was 75.3 torr and the arterial carbon dioxide tension (PaCO2) was 36.2 torr. Therefore, the ventilation\perfusion mismatch of O2‐CO2 exchange was disordered at alveoli. Data of the %VC (75.3%) and the FEV1.0 (77.2%) demonstrated the restrictive lung disorder. The computed tomography (CT) showed a honeycomb pattern at bilateral lower lungs near the pleurae. Gallium scintigraphy and the esophagography showed no abnormal findings.

Skin biopsies were taken from the left middle finger and the left lateral forearm, both of which showed sclerosis with thickened and homogenous collagen bundles in the thickened dermis. Histological features of the specimen from the lateral forearm showed that the epidermis showed irregular hyperplasia, orthohyperkeratosis, and scattered dyskeratotic cells. In the papillary dermis amorphous, eosinophilic deposits were presented (Fig. 3a). Congo‐red stain showed positive findings at papillary dermis (Fig. 3b)..

Discussion

Most interesting features in the presented case were the coexistence of miliary papules at the lateral aspects of sclerotic forearms in a patient with generalized morphea‐like scleroderma. In the papillary layer, positive findings were demonstrated by Congo‐red dye. He had no features of cutaneous amyloidosis other than on the forearms. Systemic amyloidosis was excluded by detailed investigation. Cases of localized cutaneous amyloidosis associated with systemic sclerosis [3] and sclerodermatomyositis [4] have been reported. Ogiyama et al. [3] reported 6 cases of cutaneous amyloidosis associated with progressive systemic sclerosis, and the involved sites were the upper back in all cases. To our knowledge, this case is the first report of cutaneous amyloidosis associated with generalized morphea‐like scleroderma. Although the onset of amyloidosis is unclear, amyloid deposition in localized cutaneous amyloidosis was suggested to be derived from degenerated epidermal keratinocytes through filamentous degeneration or apoptosis [5, 6]. Humphreys et al. [8] reported that amyloid deposition was made by frequent scratching in atopic dermatitis. Amyloid deposition may be caused by a similar mechanism in our case, because our patient complained of itching on the lateral forearms, face and trunk. We speculate that amyloid deposits may be induced by recurrent stimuli of the friction damage through scratching in this case.

References

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